Primary repair vs Delayed staged repair in infants with primary obstructive megaureters and their long term outcomes - A single centre experience.

BACKGROUND: There is paucity of literature in the management of infants with primary obstructive megaureters undergoing upfront primary repair with an extravesical technique of ureteral re implantation (with or without ureteral tailoring). OBJECTIVE: To compare 2 different approaches in the management of infants with unilateral primary obstructive megaureters. STUDY DESIGN: This was a retrospective analysis of a prospectively maintained data base between 2005 and 2021. Infants <1 year with unilateral primary obstructive megaureter were included. They were divided into 2 groups: those who underwent an upfront extravesical ureteric reimplantation with or without ureteral tailoring during infancy -Primary Repair (PR), and those who initially underwent a low end cutaneous ureterostomy during infancy followed by take down of ureterostomy and intravesical ureteric reimplantation after 1 year of age -Delayed staged repair (DSR). Children presenting with sepsis, in whom a diversion was imperative, were excluded. All children were followed up annually after their definitive repair with a renal ultrasound, diuretic renogram, estimated glomerular filtration rate and assessment of voiding dysfunction if present. The 1st year and 3rd year follow up details were collated and analysed. Failure was defined as persistent obstructive pattern on renogram with worsening differential renal function or presence of high grade reflux with recurrent breakthrough urinary tract infection; both of which necessitated a redo reimplantation following the definitive surgery. RESULTS: There were 18 infants in Primary repair and 16 infants in Delayed Staged Repair. Urinary tract infections was the commonest presenting symptom amongst both groups i.e. > 50%.The post operative complication rate was 11% in Primary repair and 31% in Delayed Staged Repair. One child in each of the groups (2 girls) required redo reimplantation (5.8%). At the end of the 3rd year follow up (from the definitive repair) there was significant reduction in the hydronephrosis, improvement in the differential renal function with no evidence of obstruction and improvement in the estimated glomerular filtration rate amongst all in both groups which was statistically significant i.e. p < 0.05. The success rate was 94.4% in Primary Repair and 93.75% in Delayed Staged Repair. The mean follow up was 9.7 years amongst those undergone Primary Repair and 9 years amongst those undergone Delayed Staged Repair. DISCUSSION AND CONCLUSION: Primary extravesical ureteral reimplantation may be considered as the preferred line of management of unilateral obstructed megaureters during infancy.

Thoracic Tumors in Children and their Long-Term Outcomes: A Single-Center Experience.

Aims: This study aims to review our experience in children with thoracic tumors managed by different surgical approaches, and to evaluate their long-term outcomes in relation to their functional status and quality of life. Subjects and Methods: This is a retrospective study (2011-2021). Children <18 years with tumors of the thorax (lung, mediastinum, and thoracic cage) were included. All included were diagnosed, managed, and followed up based on a departmental protocol. Children alive were followed up annually to monitor the development of chest wall/spinal deformities and assessed regarding their quality of life (Lansky play-performance scale) and pulmonary functions. Information regarding their demography, clinical presentations, diagnosis, treatment administered, outcome, and follow-up details were collated and analyzed. Results: Twenty-two children with thoracic tumors were included (2011-2021). Of which, 6/22 are benign and 16/22 are malignant lesions. About 14/22 children are alive on a regular follow-up until 2021, with a mean follow-up of 6 years (benign) and 6.25 years (malignant). About 3/22 children with malignant tumors requiring thoracotomy with rib resections developed scoliosis with a severely restrictive pattern on pulmonary functions, having a mild-to-moderate restriction of play on quality of life grading. Conclusions: Early follow-up of children who have undergone various surgical approaches for thoracic tumors based on the quality of life assessment and pulmonary function tests helps in planning early intervention if needed, especially in those who have undergone thoracotomy with rib resections, thereby improving their long-term functional status.

Metachronous Venous Malformation Masquerading as an Obscure Gastrointestinal Bleed.

Vascular malformation of the gastrointestinal (GI) tract along with metachronous lesions elsewhere in the body is extremely rare. We report an unusual case of a female child, with a history of venous malformation excision over the right leg presenting with severe iron-deficiency anemia. Although venous malformations of the small bowel are a rare cause of occult GI bleed, in the presence of cutaneous vascular lesions, there should be a high index of suspicion of a vascular malformation, along with the other causes of GI bleed in children.

Hepatic angiosarcoma developing in an infantile hemangioendothelioma: A rare case report.

Angiosarcomas are rare tumors that predominantly affect adults. Hepatic angiosarcoma in a child is extremely rare and associated with a poor prognosis. Herein, we report the pathologic features of a hepatic angiosarcoma developing in a 31/2-year-old child who had been earlier diagnosed and was being treated for hepatic hemangioendothelioma.

Chronic disseminated intravascular coagulation presenting as renal mass.

Disseminated intravascular coagulation (DIC) is a complex clinical syndrome, described as a sequential activation of the coagulation and fibrinolytic system. Trauma and sepsis are some of the known precipitating factors. We report a case of nonovert disseminated intravascular coagulation presenting as a huge renal mass in a 3-year-old child, suspected to be a Wilms' tumor. On imaging studies, it was found to be a renal hematoma. Laboratory investigations revealed an underlying chronic disseminated intravascular coagulation caused by sepsis. The child recovered with conservative treatment; follow up investigations showed resolution of renal hematoma with renal function returning to base line. Clinical presentation of Chronic DIC is variable. Laboratory investigations usually help to diagnose the condition and also to monitor the progress of the treatment. The treatment of the triggering cause is the cornerstone of the management of this condition.

Pediatric short bowel syndrome: adaptation after massive small bowel resection.

BACKGROUND: Short bowel syndrome (SBS) develops after massive small bowel resections. Patients with less than 12 cm of jejunoileum have a slim possibility of being weaned from parenteral nutrition (PN). PATIENTS AND METHODS: In a retrospective review of records of consecutive patients with SBS, 8 patients were evaluated for treatment by adaptation and weaning from PN. These included 4 patients with class I SBS (0-10 cm of small bowel), one with class II SBS (>10-25 cm), one with class III SBS (>25-50 cm), and 2 with class IV SBS (>50-75 cm). Adaptation was assessed by measuring growth in the small bowel and the ability to be weaned from PN. RESULTS: Adaptation was achieved primarily by extending the length of jejunoileum by approximately 450% over the first 2.5 years after resection and by increasing the degree of colonic fermentation and absorption of nutrients. As of July 1, 2005, all of the patients were off PN, with the exception of 2 patients with class I-A SBS: patient 3 had a remaining jejunoileum of only 2.5 cm and patient 4 had a remaining jejunoileum of 9 cm but developed eosinophilic enterocolitis. These 2 patients continued with PN on alternate months. CONCLUSIONS: Bowel growth after massive small bowel resection provides an objective parameter of adaptation and a means of predicting ability to be weaned from PN. Aggressive nutritional support makes even patients with class I SBS, whose disease was previously considered hopeless, likely candidates to achieve freedom from PN.